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Abstract：
BACKGROUND: Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is a rare disease characterized by mild clinical symptoms and non-specific imaging findings. The diagnosis of the disease depends on pathological diagnosis. However, EBV+IFDCS has a very broad spectrum of histological morphology and immune phenotypes, and its histopathological features have not been fully described by pathologists.
METHODS: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case.
CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases.
METHODS: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case.
CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases.
摘要：
背景：EB病毒阳性的炎性滤泡树突状细胞肉瘤（EBV+IFDCS）是一种罕见的疾病，其特征是轻度的临床症状和非特异性的影像学表现。该疾病的诊断取决于病理诊断。然而,EBV+IFDCS具有非常广泛的组织学形态和免疫表型，病理学家尚未完全描述其组织病理学特征。
方法：一位59岁的女性，没有明显的不适，在常规体检中发现有脾肿块。低倍镜检显示大量上皮样肉芽肿，其中观察到实质性的炎症反应。穿插在致密的炎症细胞中的是梭形或椭圆形细胞，零星分布，边界不清。在高倍放大下,这些梭形细胞具有微妙的特征：光滑透明的核膜，不显眼的小核仁，和罕见的有丝分裂图。免疫表型，梭形细胞表达CD21和CD23，Epstein-Barr编码区（EBER）原位杂交产生阳性结果。炎症环境主要由T细胞组成，少数浆细胞表达IgG4。在这种情况下，形态学和免疫组织化学结果的融合导致了EBVIFDCS的最终病理诊断。
结论：EBV+IFDCS表现为明显的肉芽肿性改变是罕见的。这种形态变异有很高的误诊风险,经常导致与其他肉芽肿性疾病混淆。准确的诊断需要全面的分析，整合免疫组织化学和原位杂交。这里介绍的案例有助于提高对EBV+IFDCS的认识和理解，目的是减少误诊和未识别的病例。
方法：一位59岁的女性，没有明显的不适，在常规体检中发现有脾肿块。低倍镜检显示大量上皮样肉芽肿，其中观察到实质性的炎症反应。穿插在致密的炎症细胞中的是梭形或椭圆形细胞，零星分布，边界不清。在高倍放大下,这些梭形细胞具有微妙的特征：光滑透明的核膜，不显眼的小核仁，和罕见的有丝分裂图。免疫表型，梭形细胞表达CD21和CD23，Epstein-Barr编码区（EBER）原位杂交产生阳性结果。炎症环境主要由T细胞组成，少数浆细胞表达IgG4。在这种情况下，形态学和免疫组织化学结果的融合导致了EBVIFDCS的最终病理诊断。
结论：EBV+IFDCS表现为明显的肉芽肿性改变是罕见的。这种形态变异有很高的误诊风险,经常导致与其他肉芽肿性疾病混淆。准确的诊断需要全面的分析，整合免疫组织化学和原位杂交。这里介绍的案例有助于提高对EBV+IFDCS的认识和理解，目的是减少误诊和未识别的病例。
